Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F24%3A00080218" target="_blank" >RIV/65269705:_____/24:00080218 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/24:00136490

Result on the web

<a href="https://ashpublications.org/bloodadvances/article/8/11/2890/515667/Serum-chemistry-profiling-and-prognostication-in" target="_blank" >https://ashpublications.org/bloodadvances/article/8/11/2890/515667/Serum-chemistry-profiling-and-prognostication-in</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1182/bloodadvances.2024012756" target="_blank" >10.1182/bloodadvances.2024012756</a>

Result language

angličtina

Original language name

Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

Original language description

Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most speci fic serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, beta 2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P &lt; .001). With regard to subvariants of AdvSM, an elevated LDH of &gt;= 260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P &lt; .001; monocytosis, r = 0.26, P &lt; .001) and the presence of an associated myeloid neoplasm (P &lt; .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308 mu g/L vs 146 mu g/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to beta 2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; &gt;= 1.5 points, 1.7 years; P &lt; .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry pro filing is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30205 - Hematology

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2024

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Blood Advances

ISSN

2473-9529

e-ISSN

2473-9537

Volume of the periodical

8

Issue of the periodical within the volume

11

Country of publishing house

NL - THE KINGDOM OF THE NETHERLANDS

Number of pages

11

Pages from-to

2890-2900

UT code for WoS article

001252795100001

EID of the result in the Scopus database

2-s2.0-85196401195